[A medico-genetic description of inhabitants of two regions of the Kransnodar Krai]. / Mediko-geneticheskoe opisanie naseleniia dvukh raionov Krasnodarskogo kraia.

The spectrum and prevalence rate of hereditary pathology in Kanevskii and Bryukhovetskii raions (districts) of Krasnodar krai (territory) were analyzed. The total size of the studied population was 145,937. The prevalence rate of monogenic hereditary pathology was estimated. This value was 1.08 +/- 0.08, 0.72 +/- 0.07, and 0.20 +/- 0.06 per 1000 people for autosomal dominant (AD), autosomal recessive (AR), and X-linked (XL) recessive diseases, respectively. Forty-two AD (158 affected persons in 82 families), 32 AR (105 affected persons in 82 families), and 6 XL disease entities (13 affected persons in 8 families) were found. A slight genetic subdivision was found in the populations of Kanevskii and Bryukhovetskii raions. However, it was not found to affect the prevalence of hereditary pathology.

[Genetico-demographic characteristics of the population from three districts of the Bryansk region]. / Genetiko-demograficheskie osobennosti naseleniia trekh raionov Brianskoi oblasti.

Results of genetic demographic investigation of three nations located in west Bryanskaya oblast (Klintsovskii, Klimovskii, and Starodubskii nations) are presented. A comparison is made with respect to a number of demographic parameters of "southern" (Krasnodar) and "northern" (Kirov) Russian populations. A low level of the Crow index, which is associated with a differential mortality rate, Im = 0.07, was established in the population studied. This fact significantly distinguishes the population of Bryanskaya oblast from other studied Russian populations. With respect to sex and age structure and the Crow index, the Bryansk population is comparable to the southern Krasnodar population, while in terms of level of isolation and inbreeding, the Bryansk population is similar to the northern Kirov population. Moreover, it was found that the Chernobyl meltdown in 1986 did not lead to any change in the genetic demographic situation in the three studied nations.

[Genetic structure and the load of hereditary diseases in five populations of Arkhangel'skaia region]. / Geneticheskaia struktura i gruz nasledstvennykh boleznei v piati populiatsiiakh Arkhangel'skoi oblasti.

A population and medical genetic investigation was performed in a number of raions in the Arkhangel' skaya oblast. Random inbreeding coefficients were 0.000358 and 0.000361 in the Vinogradovskii and Krasnoborskii raions. Malecot's local inbreeding coefficients were 0.000565 and 0.000472, respectively. The endogamy indices were 0.37 and 0.54, respectively. In the urban population, the loads of autosomal dominant, autosomal recessive, and X-linked pathology were 1.01 and 0.98 per 1000 individuals, and 0.29 per 1000 men; in the rural population, they were 1.22, 1.55, and 1.08, respectively. In the populations studied, the hereditary pathology spectrum is described.

[Genetic-demographic characteristics of highland Mari]. / Genetiko-demograficheskaia kharakteristika gornykh mariitsev.

Genetic and demographic characteristics of the population of the Gornomariiskii raion, Marii El Republic, were estimated. The length of generation was 28.16 +/- 0.45 years, the average sibship size was 2.17, Crow index was 0.687, and its components were the following: Im = 0.114 and I(f) = 0.515. the position of the population of highland Mari among other populations studied in the space of genetic and demographic characteristics is discussed.

[Population sturcture of the Gorno-Mariiskii region of the Marii El Republic]. / Populiatsionnaia struktura Gorno-Mariiskogo raiona Respubliki Marii El.

The genetic structure of the population of the Gorno-Mariiskii raion, Marii El Republic, was studied. The population consists of two major groups, highland Mari and Russians. The former constitute the majority of the rural population, and the latter, the majority of the urban one. A marked ethnicity-related marriage assortation was found. The value of random inbreeding was estimated using frequencies of surnames. It varied from 0.00015 to 0.00069 (the weighted average was 0.00026). The local inbreeding (a) for Gorno-Mariiskii raion estimated according to Malecot was 0.00029. The local inbreeding for marriages between Mari (0.0022) was five times higher than for marriages between Russians. The indices of endogamy were 0.84 in the rural population and 0.14 in the urban population. The matrix of genetic distances and its image in the form of genetic landscape suggest a regularly distorted isolation by distance and realized panmixia.

[Comparison of the population of the Krasnodar region with other Russian populations regarding genetic and demographic parameters]. / Sravnenie populiatsii Krasnodarskogo kraia s drugimi russkimi populiatsiiami po genetiko-demograficheskim parametram.

The estimates of some genetical and demographical parameters important for populational structure of the population of West part of Krasnodar province are presented. The differences in forming of population structure of districts was revealed. In general, the decrescent type of reproduction, active family planning (average family value is 2.10 +/- 0.07 when average pregnancy quantity is 6.31 +/- 0.32), significant migration activity approached rural populations of Krasnodar district to those of urban type. The migration processes was the main factor of structure population forming. When effort of natural selection was studied the decrease of index Crow was discovered in comparison with rural Russian populations Kirov and Kostroma regions. Population of Krasnodar district approaches to pan-mixture one by genetic and demographic parameters. It can be considered as a gene sample from all regions when Russian live.

[Structure and variability of hereditary disease in the Kirov Province]. / Struktura i raznoobrazie nasledstvennoi patologii v Kirovskoi oblasti.

The main purpose of this report is to present the nosological spectrum of hereditary diseases in 9 Districts of Kirov Province and to compare it with that studies earlier in other Russian populations. This comparison is undertaken in an attempt to define a "nucleus" of hereditary diseases in the Russian population studied. During this study 343 families with 546 affected were registered. The spectrum covered 55 different autosomal dominant, 14 autosomal recessive and 11 X-linked recessive hereditary disorders in the population under study. Some of these forms could be considered as common forms for the whole Russian population, because they were met in all Russian populations which were analysed. This conclusion is proved by the cluster analysis of genetic distances calculated on the basis of gene frequencies for autosomal recessive hereditary disorders.

[Load of autosomal recessive diseases in populations of Kirov Province and its relationship to consanguinity]. / Otiagoshchennost' autosomno-retsessivnoi patologiei populiatsii Kirovskoi oblasti i ee sviaz' inbridingom.

The dependence of the load of autosomal recessive disorders on a variation of random inbreeding in the populations of the 9th Districts of the Kirov Province was investigated. The values of the Fst varied between different districts from 0.00021 to 0.0011 and the values of load of AR--from 0.73 to 2.01 affected per 1000. There is a close significant correlation (r = 0.90) between the parameters in the population studied which increased under analysis of the population considering the coefficients of random inbreeding.

[Prevalence of hereditary pathologies in residents of the Kirov Province]. / Otiagoshchennost' naseleniia kirovskoi oblasti nasledstvennoi patologiei.

Medical-genetic study was carried out in the population of Kirov Province (population size about 120.000). 203 families with 334 affected with hereditary disorders were registered. The correctness of pathology classification for the inheritance type was confirmed by segregational analysis. The load of hereditary diseases in the population was: 1.25 +/- 0.06 for autosomal dominant, 1.37 +/- 0.07 for autosomal recessive and 0.22 +/- 0.06 for X-linked recessive disorders. It is suggested that variability in the values of the load of autosomal recessive disorders is determined to the large extent by genetic structure of the population.

[Variety of hereditary diseases in the Adygei Autonomous Region]. / Raznoobrazie nasledstvennykh boleznei v Adygeniskoi Avtonomnoi oblasti.

This report is one of the series of communications dedicated to medico-genetical description of the Adyg population in the autonomous national district. The peculiarities have been considered of the forms of hereditary diseases both in the Adyg and Russian populations neighbouring each other in the Adyg national district territory. It was inferred that the minimal distance between Adyg and Russian populations consists in the level of aggregation and variety of autosomal-recessive forms which depended on subdivision and the level of inbreeding in the populations studied.

[Load of hereditary diseases in the populations of the Adyg autonomic district]. / Gruz nasledstvennykh boleznei v populiatsiiakh adygeiskoi avtonomnoi oblasti.

Comparative analysis of the loads of hereditary diseases in two ethnically different populations coexisting in the Adyg national district was performed. The modes of inheritance of diseases studied were tested by segregational analysis. The results obtained demonstrated that the load of autosomal-recessive diseases in the populations of the Adyg national district is higher than that in Russian population, while the load of autosomal-dominant diseases is similar in two populations. This difference in the level of the loads appear to be connected with genetic structure of the populations studied. Regressional analysis of relations between loads and the level of inbreeding in the Adyg population showed the explicit interrelation between the load of autosomal-dominant diseases and the Fst correlation coefficient being 0.89.

[Marital and migration structure and inbreeding in the Adyg population]. / Brachno-migratsionnaia struktura i koéffitsient inbridinga v Aldygeiskoi populiatsii.

Endogamy and gametic indices for both Russian and Adyg populations living in the Adyg autonomous region of Krasnodar district were determined on different levels of territorial units: village, rural, community (a group of villagers) and rural region. Inbreeding coefficient was estimated for Adyg population and its structure analysed: a random component contributes mostly to the inbreeding coefficient (Fst = 0.00991), non-random component of the inbreeding coefficient being Fis = 0.010009, which testifies to negative marital assortativity among Adygs. Local inbreeding "a" and decline in the inbreeding "phi" coefficient at a distance from 0 to 500 km were calculated using the Malecot's formula: the coefficient "a" was found to be 0.00397, which is in good accordance with the Fst.

[Frequency criterion of choice of surname for the study of the genetic structure of the population]. / O chastotnom kriterii vybora familii dlia izucheniia geneticheskoi struktury populiatsii.

On the example of 7 populations of the regional level allowability of using surnames with frequencies exceeding 0.001 in adequate estimation of the population structure indices is shown. The correlation coefficient between genetical distances calculated with the help of all surnames in the list, and those estimated using only frequent surnames was more than 0.9 in all populations studied, values of the correlation coefficient analogous to the FST statistics of Wright being 0.76 to 0.97.

[Genetic-demographic structure of the Adyg population]. / Genetiko-demograficheskaia struktura populiatsii Adygei.

The results of comparative analysis of series of genetical and demographical parameters in two ethnically distinct populations coexisting in Adyg autonomous region of the Krasnodar district are presented. The parameters of population structure in Russians and Adygs were determined, such as sex and age distribution, ethnical marital assortativity, vital statistics and selection pressure. The analysis revealed that the populations only differ in the level of ethnical marital assortment: the coefficient of assortativity H is 2.54 and 1.55 for Adygs and Russians, respectively.

[Analysis of the structure of the urban and rural populations of the central portion of Krasnodar Krai]. / Analiz struktury gorodskikh i sel'skikh populiatsii tsentral'noi chasti krasnodarskogo kraia.

The structure of the populations in Korenovsk and Ust-Labinsk districts--sexual, age, family and migrant is described Reproduction is low in these populations. The after-reproduction age class in rural populations prevailed, which affects the ratio of recessive and dominant forms in hereditary pathology of these populations. Family planning in towns accounts for small size of the families and decrease of the role of natural selection. Gametic and endogamy indexes for these populations indicate that intensive migrant processes take place in populations studied, as shown by the level of recessive pathology. The results obtained can be used in the medical-genetic studies for this territory.

PIP: To evaluate the pattern of Mendelian hereditary pathology in Krasnodav Krai, the demographic structure of two populations from the central part of Krasnodav Krai (Korenovsk and Ust'-Labinsk regions) was studied. THe age structure of the rural population was characterized by a predominance of the 47 year-old age group (0.406 compared with 0.262 and 0.332 for 0-18 and 19-46 year old age groups, respectively). The prevalence of an adult population resulted in the prevalence of autosome dominant diseases (0.92/1000, compared with 0.56/1000 for autosome recessive diseases). Family size and pregnancy outcomes were analyzed in 170 women who were past the reproductive age. Relatively small average family size (2.06) was indicative of active family planning regulated primarily by the means of induced abortions (3.69/woman). the length of generation calculated from the data on age-dependent fertility was 29.17 years for men and 19.12 years for women. The index of total selection calculated from the Crow index for pregnancy outcomes was 0.653, which was indicative of a less marked contribution of differential mortality and differential fertility to the intensity of natural selection. The endogamy indices for the urban populations of Korenovsk and Ust'-Labinsk regions were 0.174 and 0.195, respectively, while for the rural population of Ust'-Labinsk region, the endogamy index was 0.364. The gamete index for Korenovsk region was 0.142. The calculated values of gamete and endogamy indices were indicative of intensive migration processes, weak isolation by distance, and minimal inbreeding.

[Reconstruction of genetic distance matrix]. / Rekonstruktsiia matritsy geneticheskikh rasstoianii.

A method for reconstruction of genetic distances' matrix, based on linear combination of physical distances' matrices among populations and mean sizes of the population matrices is proposed. The analogue of genetic distances' matrix obtained correlates with the matrix at the level 0.59. The reconstruction may be used for the populations of about 2-3 neighbour districts. An index xi is introduced, as a constant describing some big regions. Comparison of reconstructed matrix of genetic distances with some well-known matrices of genetic distances is performed.